Haemophilia A and haemophilia B are both X linked recessive disorders of coagulation. 1/3 of cases have no family history

  • Incidence 1 in 10 000 
  • Lack of factor VIII

Haemophilia B = Christmas disease 
  • Incidence 1 in 50 000 
  • Lack of factor IX 

Severity of the disease is classified according to the baseline level of clotting factor:
  • Severe <1% normal 
  • Moderate 1-4% normal
  • Mild 5 -50% normal 

Complications include: 
  • Haemarthrosis (70-80%) – commonest joint affected is the knee followed by elbow
  • Bleeds into muscle/soft tissue 
  • Haemophiliac arthropathy 
  • Intracranial haemorrhage 
  • Pseudotumours of bone

  • Avoid use of NSAIDs/ASA 
  • Avoid IM injections 
  • Avoid contact sports 
  • Desmopressin
  • Tranexamic acid 
  • Recombinant factor VIII or IX
  • Vaccination against hepatitis A and B

Small print gem: female carriers may have menorrhagia 

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Secret collector of interesting anonymised ECGs. Fan of the Bath Photomarathon. Lover of cream teas. [Sarah Hudson] (Your Picture)